Combination of intravenous immunoglobulin, oral prednisone, and methotrexate for managing scleromyxedema: case report and literature discussion
https://doi.org/10.4081/dr.2023.9803
HTML: 17
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
The generalized and sclerodermic form of lichen myxedematosus, known as scleromyxedema (SMX), is a chronic mucinosis that manifests cutaneously and has multiple systemic comorbidities. There are few available treatment options and no established therapeutic guidelines. We describe a 48-year-old man who had intravenous immunoglobulins (IVIg), oral corticosteroids, and methotrexate (MTX) for the treatment of SMX, monoclonal gammopathy, and arthritis. Because of its effectiveness and high level of tolerance, IVIg is the most often used first-line therapy for SMX and has been used for an increasing range of skin conditions. In our instance, better control of skin disease and extracutaneous manifestations was made possible by combining IVIg with oral prednisone and MTX. To the best of our knowledge, this is the first instance of SMX treatment that has combined therapeutic approaches with a favorable safety profile.
Rongioletti F. Lichen myxedematosus (papular mucinosis): new concepts and perspectives for an old disease. Semin Cutan Med Surg 2006;25:100-4.
Caudill L, Howell E. Scleromyxedema: a case clinically and histologically responsive to intravenous immunoglobulin. J Clin Aesthet Dermatol 2014;7:45-7.
Edward M, Fitzgerald L, Thind C, et al. Cutaneous mucinosis associated with dermatomyositis and nephrogenic fibrosing dermopathy: fibroblast hyaluronan synthesis and the effect of patient serum. Br J Dermatol 2007;156:473-9.
Cárdenas-Gonzalez RE, Ruelas MEH, Candiani JO. Lichen myxedematosus: a rare group of cutaneous mucinosis. An Bras Dermatol 2019;94:462-9.
Kreuter A, Stücker M, Kolios AG, et al. Skleromyxödem. Eine chronisch progressive Systemerkrankung [Scleromyxedema. A chronic progressive systemic disease]. Z Rheumatol 2012;71:504-14.
Rongioletti F, Merlo G, Cinotti E, et al. Scleromyxedema: a multicenter study of characteristics, comorbidities, course, and therapy in 30 patients. J Am Acad Dermatol 2013;69:66-72.
Sáez-Rodríguez M, García-Bustínduy M, López-Alba A, et al. Localized lichen myxoedematosus (papular mucinosis) associated with morbid obesity: report of two cases. Br J Dermatol 2003;148:165-8.
Rongioletti F, Parodi A, Rebora A. Papular and nodular mucinosis as a sign of lupus erythematosus. Dermatologica 1990;180:221-3.
Wong RX, Chia JC, Haber RM. Review of primary cutaneous mucinoses in nonlupus connective tissue diseases. J Cutan Med Surg 2018;22:65-70.
Rongioletti F, Rebora A. Cutaneous mucinoses: microscopic criteria for diagnosis. Am J Dermatopathol 2001;23:257 67.
Knobler R, Moinzadeh P, Hunzelmann N, et al. European dermatology forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis. J Eur Acad Dermatol Venereol 2017;31:1581-94.
Majeski C, Taher M, Grewal P, et al. Combination oral prednisone and intravenous immunoglobulin in the treatment of scleromyxedema. J Cutan Med Surg 2005;9:99-104.
Kim S, Park TH, Lee SM, et al. Scleromyxedema with multiple systemic involvement: Successful treatment with intravenous immunoglobulin. Dermatol Ther 2020;33:e13378.
Bidier M, Zschoche C, Gholam P, et al. Scleromyxoedema: clinical follow-up after successful treatment with high-dose immunoglobulins reveals different long-term outcomes. Acta Derm Venereol 2012;92:408-9.
Samuelsson A, Towers TL, Ravetch JV. Anti-inflammatory activity of IVIG mediated through the inhibitory Fc receptor. Science 2001;291:484-6.
Cokonis Georgakis CD, Falasca G, et al. Scleromyxedema. Clin Dermatol 2006;24:493-7.
Binitha MP, Nandakumar G, Thomas D. Suspected cardiac toxicity to intravenous immunoglobulin used for treatment of scleromyxedema. Indian J Dermatol Venereol Leprol 2008;74:248-50.
Xu XL, Wu Q, Zeng XS, Sun JF. Combination of betamethasone and methotrexate for the treatment of scleromyxoedema without paraproteinaemia. Eur J Dermatol 2016;26:391-3.
Mehta V, Balachandran C, Rao R. Arndt Gottron scleromyxedema: successful response to treatment with steroid minipulse and methotrexate. Indian J Dermatol 2009;54:193-5.
Efthimiou P, Blanco M. Intravenous gammaglobulin and thalidomide may be an effective therapeutic combination in refractory scleromyxedema: case report and discussion of the literature. Semin Arthritis Rheum 2008;38:188-94.
Copyright (c) 2023 the Author(s)
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.
Downloads
Citations
